Table 1

Baseline characteristics (N = 89)

CharacteristicNo. of patientsReference valueCohort value
No.%
Age at diagnosis, y  —   
 Median   71.6 
 Range   42.9-91.0 
Age at onset of symptoms, y  —   
 Median   64.6 
 Range   40.6-82.6 
Male sex  — 35 39 
Race     
 White  — 84 94 
 Black  — 
 Native American  — 
 Other  — 
 Unknown  — 
Duration of follow-up, mo  —   
 Median   66.5 
 Range   0.1-348.7 
Hemoglobin, g/dL     
 Median 85  10.2 
 Range  13.5-17.5 6.2-17.7 
Lactate dehydrogenase, μkat/L     
 Median 69  4.4 
 Range  2.0-3.7 2.0-16.5 
Bilirubin, μmol/L     
 Median     
 Range     
Total 73 1.7-17.1 35.9 3.4-114.6 
Direct 49 0-5.1 6.8 1.7-188.1 
Coombs testing, % positive 71 Negative 67 94 
Monospecific anti-C3d, negative anti-IgG 46 Negative 30 65 
Monospecific anti-C3d, positive anti-IgG 46 Negative 13 28 
Monospecific anti-IgG, negative anti-C3d 46 Negative 
Polyspecific, negative anti-C3d, negative anti-IgG 46 — 
Monospecific anti-C3d, no other antibody testing performed — 100 
Polyspecific testing, no monospecific testing performed 18 — 18 100 
Cold agglutinin titer* 79    
 1970-2007  Negative   
  Median   1:215-216 
  Range   1:22-1:221 
 2008-2012  Negative   
  Median   >1:29 
  Range n = 19    
CharacteristicNo. of patientsReference valueCohort value
No.%
Age at diagnosis, y  —   
 Median   71.6 
 Range   42.9-91.0 
Age at onset of symptoms, y  —   
 Median   64.6 
 Range   40.6-82.6 
Male sex  — 35 39 
Race     
 White  — 84 94 
 Black  — 
 Native American  — 
 Other  — 
 Unknown  — 
Duration of follow-up, mo  —   
 Median   66.5 
 Range   0.1-348.7 
Hemoglobin, g/dL     
 Median 85  10.2 
 Range  13.5-17.5 6.2-17.7 
Lactate dehydrogenase, μkat/L     
 Median 69  4.4 
 Range  2.0-3.7 2.0-16.5 
Bilirubin, μmol/L     
 Median     
 Range     
Total 73 1.7-17.1 35.9 3.4-114.6 
Direct 49 0-5.1 6.8 1.7-188.1 
Coombs testing, % positive 71 Negative 67 94 
Monospecific anti-C3d, negative anti-IgG 46 Negative 30 65 
Monospecific anti-C3d, positive anti-IgG 46 Negative 13 28 
Monospecific anti-IgG, negative anti-C3d 46 Negative 
Polyspecific, negative anti-C3d, negative anti-IgG 46 — 
Monospecific anti-C3d, no other antibody testing performed — 100 
Polyspecific testing, no monospecific testing performed 18 — 18 100 
Cold agglutinin titer* 79    
 1970-2007  Negative   
  Median   1:215-216 
  Range   1:22-1:221 
 2008-2012  Negative   
  Median   >1:29 
  Range n = 19    
*

Ten patients had records from outside laboratories indicating a positive cold agglutinin titer.

The method of reporting cold agglutinin titers changed in 2008; all results obtained after this date were reported as positive or negative for a titer greater than 1:29. Since 2008, all patients for whom cold agglutinin titers were measured had outcomes greater than 1:29.

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